Zobrazeno 1 - 10
of 41
pro vyhledávání: '"Geraldine Kent"'
Autor:
Yana Urman, John Zeind, Michael Laposata, Geraldine Kent, Munir M. Zaman, Mario Ollero, Steven D. Freedman, Juan G. Alvarez, Charlotte Andersson, Paola G. Blanco
Publikováno v:
Metabolism. 55:1192-1200
An association has been reported between alterations in fatty acid metabolism and cystic fibrosis (CF). We hypothesized that these alterations are specific for a particular lipid component(s) and are the result of a specific metabolic defect. The dif
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a8c12c0d8466a149bdac7d775fa5e260
https://doi.org/10.1016/j.metabol.2006.05.002
https://doi.org/10.1016/j.metabol.2006.05.002
Publikováno v:
The American Journal of Pathology. 164:1481-1493
The lack of an appropriate animal model with multiorgan pathology characteristic of the human form of cystic fibrosis has hampered our understanding of the pathobiology of the disease. We evaluated multiple organs of congenic C57BL/6J cystic fibrosis
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::cd4eff7a8eb7cc607d2461e0a5ba7d48
https://doi.org/10.1016/s0002-9440(10)63234-8
https://doi.org/10.1016/s0002-9440(10)63234-8
Autor:
Jim Hu, Yu Hua Chow, Geraldine Kent, Umadevi S. Sajjan, Bernard Martin, David R. Koehler, A. Keith Tanswell, Colin McKerlie, Janet F. Forstner
Publikováno v:
Proceedings of the National Academy of Sciences. 100:15364-15369
We developed a helper-dependent adenoviral vector for cystic fibrosis lung gene therapy. The vector expresses cystic fibrosis transmembrane conductance regulator ( Cftr ) using control elements from cytokeratin 18 . The vector expressed properly loca
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::11c0c8a3389e9a24a68c656384d9b894
https://doi.org/10.1073/pnas.2436478100
https://doi.org/10.1073/pnas.2436478100
Autor:
Carola Sjolin, Brent Steer, Annie Lu, Geraldine Kent, Gregory P. Downey, Yi Jun Wu, Janet F. Forstner, Vera Cherapanov, George Thanassoulis, Colin McKerlie, Uma Sajjan, Ori D. Rotstein
Publikováno v:
Infection and Immunity. 69:5138-5150
Progressive pulmonary infection is the dominant clinical feature of cystic fibrosis (CF), but the molecular basis for this susceptibility remains incompletely understood. To study this problem, we developed a model of chronic pneumonia by repeated in
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::08adc9fc5fe20a9434f05e586b7b528e
https://doi.org/10.1128/iai.69.8.5138-5150.2001
https://doi.org/10.1128/iai.69.8.5138-5150.2001
Publikováno v:
Journal of Medical Microbiology. 49:875-885
The Burkholderia cepacia complex consists of at least five well-documented bacterial genomovars, each of which has been isolated from the sputum of different patients with cystic fibrosis (CF). Although the world-wide prevalence of this opportunist p
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c316490e111a70b04c6d936ea3decde4
https://doi.org/10.1099/0022-1317-49-10-875
https://doi.org/10.1099/0022-1317-49-10-875
Publikováno v:
Pediatric Research. 45:482-488
We evaluated pancreatic enzyme secretory response to secretagogues (cAMP- and Ca2+-mediating) involved in exocytosis and in chloride channel activation in an exon 10 knockout cystic fibrosis (CF) mouse model. Experiments were performed in isolated pa
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::296b4446c2740077e3ba5ba3d05c2109
https://doi.org/10.1203/00006450-199904010-00005
https://doi.org/10.1203/00006450-199904010-00005
Autor:
Peter R. Durie, Christine E. Bear, Ling-Jun Huan, Keith A. Tanswell, Canhui Li, Mohabir Ramjeesingh, Geraldine Kent, Katalin Gyömörey, Michael Wilschanski, Cameron Ackerley, Yanchun Wang, Ernest Cutz
Publikováno v:
Human Gene Therapy. 9:521-528
Cystic Fibrosis (CF) is caused by mutations in the CF gene that lead, for the most part, to mislocalization of the protein product, the cystic fibrosis transmembrane conductance regulatory (CFTR). CFTR is a chloride channel normally situated in the a
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c9465c0e87d0c65297dc4ef2b0353e95
https://doi.org/10.1089/hum.1998.9.4-521
https://doi.org/10.1089/hum.1998.9.4-521
Publikováno v:
Pediatric Research. 40:242-249
We investigated the development of the exocrine pancreas in Cftr -/- mice in comparison with age-matched littermates (Cftr +/+ , Cftr +/- ) up to 100 d postnatally. Controls were weaned either to mouse chow or a liquid diet ; Cftr -/- mice were weane
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::59a3fabbd10542f5c3f48a7b60d5c360
https://doi.org/10.1203/00006450-199608000-00009
https://doi.org/10.1203/00006450-199608000-00009
Autor:
Mary Oliver, Geraldine Kent, John R. Riordan, Janet F. Forstner, J. Kevin Foskett, Helena Frndova, Peter R. Durie, Gordon G. Forstner, Dean H. Percy, Manuel Buchwald
Publikováno v:
Scopus-Elsevier
Mouse models for cystic fibrosis (CF) with no CFTR function (Cftr-/-) have the disadvantage that most animals die of intestinal obstruction shortly after weaning. The objective of this research was to extend the lifespan of CF mice and characterize t
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::73d913f1b9b18a241c74a5a3f22f12ab
https://doi.org/10.1203/00006450-199608000-00008
https://doi.org/10.1203/00006450-199608000-00008
Autor:
Lap-Chee Tsui, Geraldine Kent, Peter R. Durie, Richard Rozmahel, Christine E. Bear, Satti Beharry, Canhui Li, M. Wilschanski
Publikováno v:
Biochemical and Biophysical Research Communications. 219:753-759
The Cftr (Cystic Fibrosis Transmembrane Conductance Regulator) gene codes for an epithelial chloride (C1) channel essential for fluid secretion into the respiratory and gastrointestinal tract and from exocrine glands. Mice lacking CFTR function due t
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::49ac5b25bc9fa4d7bda0e3e469df8e30
https://doi.org/10.1006/bbrc.1996.0306
https://doi.org/10.1006/bbrc.1996.0306